IMMUNODEFICIENCY IN CHILDREN
Consider Primary Immunodeficiency – if
- Recurrent infections or unusual or persistent infection
- Life-threatening Infections
- Blood cell counts low or persistently high
TYPES of primary deficiency-
a) ANTIBODY PRODUCTION DEFECTS-
example
- X-Linked Agammaglobulinemia
- Common Variable Immunodeficiency (CVID)
- X-Linked or Autosomal Hyper IgM Syndrome Hyper IgM
- Selective IgA Deficiency
b) CELLULAR OR COMBINED DEFECTS
- Severe Combined Immunodeficiency
- Di- George Syndrome
- Ataxia Telangiectasia
- Wiskott-Aldrich Syndrome
c) PHAGOCYTIC CELL IMMUNE DEFECTS
- Leukocyte Adhesion Defect
- Chronic Granulomatous Disease
- Chediak Higashi Syndrome
- Cyclic Neutropenia
d) COMPLEMENT DEFECTS
- C1 Esterase Inhibitor Deficiency
- Complement Component Deficiencies
MANAGEMENT OF ANTIBODY PRODUCTION DEFECTS
TREATMENT- Is by Immunoglobulin replacement
MANAGEMENT OF CELLULAR OR COMBINED DEFECTS
MANAGEMENT OF PHAGOCYTIC CELL DEFECTS
MANAGEMENT OF COMPLEMENT DEFECTS